Journal of Medical Sciences and Health

Introduction

Sarcomas of the breast are rare, malignant tumors arising from the mesenchymal tissue of the mammary gland.[1] Breast sarcomas are broadly divided into three groups, (a) malignant phyllodes tumors, (b) sarcomas arising in breast that has received irradiation, and (c) primary breast sarcomas (PBS).[2] The incidence of PBS is reported to be as low as < 1% of all breast masses.[3] Owing to the rarity of occurrence, no clear cut directives exist for early diagnosis and management of PBS. Size of the tumor at the time of diagnosis is an important factor in predicting survival. Surgical resection with clear margins is also a very important predictor of outcome.[4] Adjuvant radiotherapy and chemotherapy are also important in the management algorithm of breast sarcomas.[5]
We present a case of an aggressive PBS in a 19-year- old female.

Case Report

Figure 1: Photograph showing fungating mass in the right breast, replacing the entire breast

Figure2: Photograph showing Latissimus dorsi flap reconstruction of right breast

Figure 3: Hematoxylin and eosin staining of right breast sarcoma showing spindle shaped cells arranged in whorls, sheets and fascicles (H & E, ×200)

Discussion

Sarcomas arising as a primary tumor are extremely rare.[3] Sarcomas arising in the breast are more common after exposure to radiation.[6] A variety of subtypes of sarcomas are seen, most common ones being -malignant phyllodes and angiosarcoma.[7] Owing to the rarity of its occurrence and fewer anecdotal reporting, the guidelines for management of sarcomas are vague. Early diagnosis, clear surgical margins of at least 1 cm and tumor size at the time of resection are the most important indicators of outcome.

The commonly used approach of diagnosis of breast lumps with clinical exam and FNAC may not be enough to identify PBS. A high index of suspicion is required while evaluating breast lumps that are recurrent or atypical. PBS is easily confused with fibro adenoma on cytological analysis.[2] The consequences of such false diagnosis can be catastrophic, as exemplified in the present case. Tissue diagnosis by core biopsy supersedes FNAC in accurately identifying sarcomas.[8] Mammography or ultrasound imaging of the breast can have non- specific features and may not be conclusive.[9,10] Magnetic resonance imaging, however, is better at identifying suspicious lesions. Tumors are usually lobulated and have “washed out” appearance, - i.e., display rapid enhancement.[11]

Surgical resection is still the best treatment option for PBS.[3] A tumor size < 5 cm at the time of resection has a better prognosis.[3,7] Clear surgical margin of at least 1 cm from tumor also is a favorable factor for improved outcomes.[3,7] Wide local excision is the procedure of choice, while large tumors may require a simple mastectomy to ensure adequate clearance of margin.[2] Axillary lymph node dissection is not warranted since sarcomas being mesenchymal in origin disseminate hematogenously.[2] Enlarged axillary nodes are usually reactive enlargements and rarely due to spread of the tumor. Complete axillary dissection has not been shown to be beneficial.[12,13] Adjuvant chemotherapy and radiotherapy are both a part of the treatment algorithm. Radiation to the affected site has been shown to improve survival rates in a study done at Toronto.[14] The beneficial effects of chemotherapy are advocated by some studies.[13]

Despite advances in management, early and appropriate diagnosis is vital in attaining better outcomes in PBS. Delay in diagnosis coupled with aggressiveness of the tumor make a lethal combination and must be avoided.

Conclusion

PBS is rare entities and need a high index of suspicion for diagnosis. Fine-needle aspiration studies alone are insufficient to exclude sarcomas. Early diagnosis and surgical intervention may affect the outcome of the disease. Aggressive tumors rapidly progress and have a poor survival rate, despite therapy.